"La picnodisostosis es un sindrome genetico raro caracterizado por presentar una amplia variedad de desordenes a nivel del tejido oseo, tales como estatura baja, osteopetrosis, acroosteolisis de las falanges terminales, alteraciones de la forma y tamano de los huesos craneofaciales, persistencia de las fontanelas y falta de cierre de la suturas craneales. Se presentan los hallazgos clinicos y radiograficos identificados en una paciente con este sindrome quien ademas tenia un cuadro de osteomielitis del maxilar inferior. Las manifestaciones en la cabeza fueron multiples y variadas, dentro de las cuales destacaron: platibasia, severa hipoplasia de senos paranasales, hipoplasia del maxilar superior e inferior, ausencia del angulo mandibular, hipercementosis, estrechamiento de la camara pulpar y conductos radiculares y erosion de la cresta alveolar sin evidencia clinica de periodontitis. La imagen radiografica de la cabeza correspondia a la forma de una pera. El estudio histologico de la cresta alveolar mostro hueso compacto con una disminucion del numero de osteonas, estas ademas tenian forma asimetrica y algunas presentaban marcada obliteracion de los conductos de Havers. Se agregan nuevas caracteristicas fenotipicas y se discute los aspectos de prevencion y manejo de las complicaciones infecciosas de los maxilares que se pueden presentar en este sindrome. (AU)."@es
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